ABSTRACT
El penfigoide de la membrana mucosa es una enfermedad autoinmune, que afecta a los diferentes estratos de la mucosa y la piel, y se caracteriza por lesiones vesiculo-ampollosas polimórficas. Presentando combinaciones variables de lesiones orales, oculares, cutáneas, genitales, nasofaríngeas, esofágicas y laríngeas. Su relación con las neoplasias malignas es poco conocida y sigue siendo controvertida. El presente artículo describe el manejo multidisciplinario de una paciente de 46 años de edad que desarrolló un penfigoide de membrana mucosa a nivel de la cavidad oral. Esta patología estuvo asociada a un carcinoma de pulmón, y posteriormente presentó alteraciones multiorgánicas. El tratamiento fue realizado por las especialidades de dermatología, medicina interna, cirugía general, oftalmología, estomatología, cirugía oral y maxilofacial, dicho tratamiento se llevó a cabo por etapas y tuvo una duración de 2 meses aproximadamente, logrando la resolución de las lesiones que presentaba la paciente. Consideramos que se abre una puerta para futuras investigaciones que confirman la relación entre el penfigoide de la membrana mucosa y el carcinoma de pulmón.
Mucous membrane pemphigoid is an autoimmune disease that affects different layers from mucosa and skin, characterized by polymorphic vesicular-bullous lesions, presenting variable combinations of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions. Its relationship to malignant neoplasms is poorly understood and remains controversial. This article describes the multidisciplinary management of a 46-year-old patient who developed mucous membrane pemphigoid at the oral cavity level. This pathology was associated with lung carcinoma, and later presented multiorgan alterations, for which a treatment was carried out with the specialties of dermatology, internal medicine, general surgery, ophthalmology, stomatology, oral and maxillofacial surgery, said treatment was carried out in stages and lasted for approximately 2 months, achieving the resolution about lesions that patient presented. We believe that a door opens for future research confirming the relationship between mucous membrane pemphigoid and lung carcinoma.
ABSTRACT
Skin diseases manifesting as desquamative gingivitis (DG) can be divided into recurrent DG-and chronic DG-related skin diseases,including oral lichen planus,mucosal pemphigoid,pemphigus vulgaris and so on.A thorough medical history,detailed oral and histopathological examinations and serum immunological tests can be helpful for correct diagnosis of DG-related skin diseases.The treatment of DG-related skin diseases includes topical and systemic therapies.It is necessary to individualize treatment protocols due to treatment response.During the treatment of DG,oral hygiene should be strengthened,secondary fungal and bacterial infections should be avoided,and attention should be paid to the protection of oral cavity and periodontal tissues.
ABSTRACT
BP180-related autoimmune blistering diseases include bullous pemphigoid,lichen planus pemphigoides,linear IgA bullous dermatosis,pemphigoid gestationis and cicatricial pemphigoid.There are multiple autoantibody-reactive sites on the extracellular region of BP180.Current studies show that there is heterogeneity in the autoimmune blistering disease-related target sites on BP 180,and different clinical manifestations of the same disease are related to the heterogeneity of target sites.However,further studies and analysis are still needed for the mechanism of the heterogeneity.
ABSTRACT
ABSTRACT Rosacea is a chronic, progressive disease of unknown cause affecting the eye and the facial skin. Ocular rosacea is often underdiagnosed if the ophthalmologist does not inspect the patient's face adequately during the ocular examination. Severe ocular complications and blindness can occur if the treatment is delayed because of non-diagnosis of the rosacea. Here, we present a case of ocular rosacea in a 78-year-old Caucasian woman. Based on the ocular lesions, which preceded cutaneous involvement, she was misdiagnosed as having ocular cicatricial pemphigoid initially. This case emphasizes the difficulty in diagnosis when ocular findings precede those of skin manifestations, and rosacea should be kept in mind in the differential diagnosis of chronic cicatrizing conjunctivitis.
RESUMO A rosácea é uma doença crônica e progressiva de causa desconhecida que afeta o olho e a pele do rosto. A rosácea ocular é muitas vezes pouco diagnosticada se o oftalmologista não inspecionar adequadamente o rosto do paciente durante o exame ocular. Podem ocorrer complicações oculares graves e cegueira se o tratamento for adiado devido ao não diagnóstico da rosácea. Um caso de rosácea ocular em uma mulher caucasiana de 78 anos de idade é apresentado. Com base nas lesões oculares, que precederam o envolvimento cutâneo, ela foi mal diagnosticada como penfigóide cicatricial ocular inicialmente. Este caso enfatiza a dificuldade de diagnóstico quando os achados oculares precedem aqueles das manifestações da pele e um diagnóstico de rosácea deve ser mantido em mente no diagnóstico diferencial da conjuntivite cicatrizante crônica.
Subject(s)
Humans , Female , Aged , Pemphigoid, Benign Mucous Membrane/diagnosis , Rosacea/diagnosis , Conjunctivitis/diagnosis , Diagnosis, Differential , Diagnostic ErrorsABSTRACT
Introdução: Gengivite descamativa (GD) se refere a uma manifestação clínica associada com diversas doenças mucocutâneas. Suas causas mais comuns são penfigóide das membranas mucosas (PMM), pênfigo vulgar (PV) e líquen plano oral (LP). A diagnose específica é melhor estabelecida através de avaliação histopatológica e de imunofluorescência. Objetivos: Examinar casos de gengivite descamativa utilizando microscopia confocal a laser e comparar os achados com aqueles encontrados na gengiva normal. Além disso, comparar os achados de microscopia confocal da gengivite descamativa com os da histopatologia convencional das lesões biopsiadas a fim de estabelecer critérios para este método diagnóstico não invasivo. Método: Doentes com manifestações clínicas de gengivite descamativa foram incluídos, totalizando quarenta e três casos. A microscopia confocal foi realizada na gengiva de um indivíduo saudável e nas lesões gengivais. Todas as lesões sem exame histopatológico prévio foram biopsiadas a fim de permitir uma correlação entre a microscopia confocal e a histopatologia. Resultados: O exame de microscopia confocal das lesões suspeitas de penfigóide das membranas mucosas revelou uma separação ao nível da junção dermo-epidérmica, preenchida por pequenas estruturas brilhantes, interpretadas como hemáceas. Os aspectos histopatológicos e de imunofluorescência confirmaram o diagnose. Para os casos de pênfigo vulgar, os achados da microscopia confocal foram de fenda intraepitelial com células arredondadas interpretadas como queratinócitos acantolíticos. Hiperqueratose e espongiose, associadas com infiltrado inflamatório, caracterizado por células pequenas e brilhantes permeando a estrutura intraepitelial de queratinócitos conhecida como favo de mel foram vistos no líquen plano. Estruturas arredondadas pouco brilhantes, interpretadas como queratinócitos necróticos, e estruturas estelares também pouco brilhantes, interpretadas como melanófagos, foram encontrados...
Background: Desquamative gingivitis refers to a clinical manifestation associated with several mucocutaneous disorders. The most common are mucous membrane pemphigoid, pemphigus vulgaris and lichen planus. Their specific diagnosis is better established by histopathological and immunofluorescence evaluation. Objective: To examine cases of desquamative gingivitis using reflectance confocal microscopy and compare the findings with those of normal gingiva. Moreover, confocal microscopy findings in desquamative gingivitis were compared to conventional histopathology of the biopsied lesions, in order to establish criteria for this non-invasive diagnostic technique. Methods: Patients with clinical manifestations of desquamative gingivitis were included, totalizing forty-three cases. Reflectance confocal microscopy was performed the gingival of a healthy person and on gingival lesions. All lesions were biopsied in order to perform a reflectance confocal microscopy- histopathologic correlation. Results: Reflectance confocal microscopy exam of the gingival lesions suspected of mucous membrane pemphigoid revealed a separation at the level of dermal-epidermal junction, filled with small bright structures interpreted as blood cells. Histopathological and immunofluorescence aspects confirmed the diagnosis. For pemphigus vulgaris, reflectance confocal microscopy aspects were of intraepithelial cleft with round detached cells interpreted as acantholytic keratinocytes, similar to the histopathological features. Hyperkeratosis and spongiosis associated with infiltration of inflammatory cells, recognized as small bright cells intermingling the honeycomb keratinocyte epithelial structure, were seen in lichen planus. Mild bright round structures interpreted as necrotic keratinocytes and mild bright stellate structures, interpreted as melanophages in the dermis were also seen. These features were present in histopathology, confirming the diagnosis of lichen...
Subject(s)
Humans , Male , Female , Adolescent , Young Adult , Adult , Middle Aged , Aged , Aged, 80 and over , Gingival Diseases , Lichen Planus, Oral , Microscopy, Confocal , Pemphigoid, Benign Mucous Membrane , PemphigusABSTRACT
BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect ...
Subject(s)
Female , Humans , Male , Autoimmune Diseases/diagnosis , Fluorescent Antibody Technique/methods , Skin Diseases, Vesiculobullous/diagnosis , Autoimmune Diseases/immunology , Immunoglobulin A/immunology , Immunoglobulin G/immunology , Reproducibility of Results , Retrospective Studies , Sensitivity and Specificity , Skin Tests , Skin Diseases, Vesiculobullous/immunologyABSTRACT
Objective To analyze the clinical features and treatment of 68 cases of mucous membrane pemphigoid (MMP).Methods Clinical data were collected from 68 patients with MMP at the Department of Dermatology,Peking University First Hospital,between August 1987 and October 2012.Skin manifestations,histopathological and immunological findings were studied,with an emphasis on treatment regimens.Results The two most frequently involved sites were oral (67/68,98.5％) and conjunctival (23/68,33.8％) mucosa in patients with MMP.Immunological examinations included direct immunofluorescence test,indirect immunofluorescence test and enzyme-linked immunosorbent assay,with a positivity rate of 50％ (8/16),20.5％ (8/39) and 53.7％ (22/41) respectively.According to lesion distribution and disease severity,patients were given local therapy (n =5) or low to moderate dose of glucocorticoids (n =55,0.4-0.5 mg/kg or 30 mg per day).The condition was controlled until the dose of glucocorticoids reached 50 mg/d in three patients with cutaneous,oral,ocular,and other mucosal involvement.The time to onset of action of glucocorticoids at the controlling dose was (11.80 ± 5.88) days,and the duration of administration of glucocorticoids at this dose varied from 0.23 to 12 months (average,3.06 ± 2.84 months).Fifteen patients were almost cured,and four patients completely cured.Of the 15 patients almost cured,the time required for a 50％ reduction in the dose of glucocorticoids was (13.29 ± 5.76) months,and that required for the control of MMP was (17.33 ± 7.71) months.The dose of glucocorticoids was decreased to 76.5％ of the controling dose at 6 months after the control of MMP,and 58.1％ of that at 12 months.Oral candidiasis occurred in three patients during the treatment.Conclusions The diagnosis of MMP is mainly based on typical clinical and histopathological findings,and current immunological examinations are insufficiently sensitive.Usually,low to moderate dose of systemic corticosteroids combined with topical therapy can lead to satisfactory treatment outcomes.
ABSTRACT
O penfigoide de membranas mucosas é entidade nosológica encarada como um fenótipo, que engloba várias dermatoses autoimunes com lesões bolhosas subepidérmicas, ocorrendo predominantemente nas membranas mucosas, com êxito cicatricial. O acometimento esofágico no penfigoide de membranas mucosas é raro e observado em pacientes com lesão disseminada. As alterações mais comuns são múltiplas membranas ou constrições esofagianas. No presente relato, os autores apresentam paciente com PMM sem lesões cutâneas e estenose esofágica grave, que entrou em remissão após uso de imunoglobulina venosa.
Mucous membrane pemphigoid (MMP) is a rare nosological entity. MMP consists of a clinical phenotype in which several autoimmune subepidermal bullous diseases are classified. It occurs predominantly in the mucous membranes and usually results in scarring. Esophageal involvement in MMP is rare and is generally seen in patients in whom lesions are widespread. The most common alterations are multiple esophageal membranes or strictures. In the present case, the authors report on a patient with MMP without any skin lesions and with severe esophageal strictures who went into remission following use of intravenous immunoglobulin.
Subject(s)
Aged , Female , Humans , Esophageal Stenosis/etiology , Pemphigoid, Benign Mucous Membrane/complications , Esophageal Stenosis/diagnosis , Esophageal Stenosis/drug therapy , Glucocorticoids/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapy , Prednisone/therapeutic use , Severity of Illness IndexABSTRACT
O presente projeto de pesquisa tem como objetivo avaliar a prevalência, os aspectos clínicos, a severidade e os fatores de risco das manifestações gengivais de doenças de origem imunológica, além de avaliar a efetividade de medidas de controle da placa bacteriana na melhora dos sinais e sintomas de pacientes que apresentam líquen plano bucal com envolvimento gengival. Para isso, foram elaborados três estudos. No primeiro deles, com o objetivo de avaliar a epidemiologia da gengivite descamativa em um Serviço de Medicina Bucal, foram selecionados casos de líquen plano, penfigóide das membranas mucosas, pênfigo vulgar, lupus eritematoso e eritema multiforme com manifestações gengivais, atendidos no Serviço de Medicina Bucal da Faculdade de Odontologia de Araraquara - Unesp, no período de janeiro de 1995 a dezembro de 2004. Dos 4.776 prontuários avaliados, 48 apresentaram lesões de manifestações gengivais, sendo 68.8% mulheres, com idade média de 43.3 anos. As doenças mais comuns encontradas na amostra foram o líquen plano, o penfigóide das membranas mucosas e o pênfigo vulgar. A maioria dos pacientes avaliados apresentava sintomatologia dolorosa (83.3%) e apenas 35.4% dos casos apresentaram lesões restritas à gengiva. Nossos resultados contribuíram para mostrar a importância do diagnóstico precoce e consequentemente do adequado tratamento das manifestações gengivais associadas a doenças de origem imunológica. O Segundo estudo, também retrospectivo, objetivou avaliar a prevalência de lesões gengivais em portadores de líquen plano bucal, bem como os aspectos e fatores de risco associados, a partir de dados de prontuários de pacientes atendidos no mesmo Serviço, no período compreendido entre janeiro de 1995 e dezembro de 2009. Dos 9.475 prontuários analisados, 84 apresentaram...
This research project aims to assess the prevalence, clinical aspects, severity and risk factors of gingival disease of immune origin, and to evaluate the effectiveness of control plaque in the improvement of signs and symptoms of patients lichen planus presenting with gingival involvement. For this, we prepared three studies. In the first, in order to assess the epidemiology of desquamative gingivitis in a Department of Oral Medicine, were selected cases of lichen planus, oral pemphigoid, pemphigus vulgaris, lupus erythematosus and erythema multiforme in the gingiva, the Service's Oral Medicine Faculty of Dentistry of Araraquara - UNESP, from January 1995 to December 2004. Of 4,776 records evaluated, 48 had lesions associated with desquamative gingivitis, and 68.8% women, mean age of 43.3 years. The most common disease found in the sample were lichen planus, oral pemphigoid and pemphigus vulgaris. Much of the patients had painful symptoms (83.3%) and only 35.4% of cases had lesions limited to the gingiva. Despite the small sample size, the results helped show the importance of early diagnosis and consequently the appropriate treatment of diseases associated with desquamative gingivitis. The second study, also retrospective study aimed to assess the prevalence of gingival lesions in patients with lichen planus, as well as the aspects and associated risk factors in medical records of patients attending the same service in the period between January 1995 and December 2009. Of 9,475 charts analyzed, 84 had lichen...